CORRESPONDENCE
A Mass Under Parotid Gland: A Patient With Kimura Disease To the Editor: Recently, we treated a 52-year-old middle-aged man with a mass under the left parotid gland. The size was equivalent to a soybean 3 months ago, constantly growing and reaching recently to a size of an egg yolk with considerable pain. After surgical intervention, the biopsy revealed Kimura disease (Fig. 1). Without a pathologic report, we can only exclude metastatic tumors, blood disease, and mycobacterium tuberculosis infection. This is the first case of Kimura disease diagnosed in our hospital, which reminds us to pay more attention to it. Hence, we did the following work. Kimura disease is a chronic inflammatory disease of unknown etiology with a good prognosis. Clinically, patients present with head and neck neoplasm, which tends to enlarge progressively, involving regional lymph nodes or parotid gland metastasis. This disease is mostly found in Asians, mostly young men (the patient in this case is a middle-aged man). The ratio of men-towomen prevalence is 6:1 to 10:1, seen in all ages. This disease is most common in unilateral head and neck subcutaneous mass. The lump often has a deeper location. It can involve large salivary glands, especially the parotid gland and the submandibular gland, and nearly half of all cases of regional lymph node enlargement.1 The pathogenesis of Kimura disease is still unclear. Some scholars suggest that it is a kind of immune-mediated inflammation disease associated with autoimmune factors.2 The pathogenesis includes the following: autoimmune dysfunction, allergy, tumor proliferation and insect bites, parasites, Candida infection, and the like.1 The disease may be associated with nephrotic syndrome, skin lichen amyloidosis, mouth ulcers, refractory hypertension, and other diseases,3 whereas some evidence of its pathogenesis may be associated with abnormal systemic immune response. Half of the patients have pruritic skin lesions, but this is not the main reason why most patients seek medical treatment. Most patients seek medical treatment because of the increasing size of the lump. The disease's most important criteria are biochemical abnormalities such as elevated eosinophil counts and elevated serum immunoglobulin E levels, whereas 12% to 16% of patients have proteinuria, lymph nodes mass, or lumps preceding kidney failure by several months.4 Clinical manifestations of the disease should be the craniocervical neoplastic inflammation with differential diagnosis including lymphoma, eosinophilic granuloma, histiocytosis, metastatic cancer, parasitic infections, allergic diseases, and cervical lymph node tuberculosis.5 Kimura disease's treatments include surgery, radiation therapy, and low-dose glucocorticoid therapy. After a simple partial tumor resection, there is a recurrence rate of 40%, which leads to the consideration of postoperative radiotherapy or hormonal therapy. The symptoms can be controlled by hormonal therapy alone, but subsequent hormonal dose tapering can induce relapse. Therefore, the addition of radiotherapy and immunosuppressive agents can give a better outcome.6 Recently, Sun et al7 proposed a treatment with imatinib because of its inhibitory effect on the proteintyrosine kinases platelet-derived growth factor receptor and c-kit gene reported in the hypereosinophilic syndrome. Sun et al7 and Buchdunger et al8 found a positive expression of c-kit gene and platelet-derived growth factor receptor-α in patients with Kimura disease as well and concluded that imatinib could therefore have a beneficial effect on Kimura disease.
FIGURE 1. Eosinophilic infiltration and proliferation of postcapillary venules can be seen. No evidence of malignancy or metastatic deposits is seen (100 magnification [A, B] and 400 magnification [C, D]).
Kimura disease has a rare occurrence. Most clinicians lack awareness of this disease, and this could easily lead to misdiagnosis. This disease should be considered in patients with craniocervical neoplasm, slow onset of disease, elevated eosinophils count, and serum immunoglobulin E in biochemical analysis, especially if the values are significantly increased. Afterward, proceed to a pathologic examination for confirmation.
Yanxin Gong, MM Department of Thyroid Surgery the Second Affiliated Hospital of Dalian Medical University Liaoning Province, China Yongfu Zhao, MD Department of Thyroid Surgery the Second Affiliated Hospital of Dalian Medical University Liaoning Province, China [email protected]
REFERENCES 1. Bonils P, Moya-Plana A, Badoual C, et al. Intraparotid Kimura disease. Eur Ann Otorhinolaryngol Head Neck Dis 2013;130:87–89 2. Hallam LA, Mackinlay GA, Wright AM. Angiolymphoid hyperplasia with eosinophilia: possible aetiological role for immunisation. J Clin Pathol 1989;42:944–949 3. Chartapisak W, Opastirkul S. Steroid-resistant nephrotic syndrome associated with Kimura's disease. Am J Nephrol 2002;22:38 4. Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14:486–488 5. Bangal SV, Chitgopekar RP, Gupta AK. Orbital extension of supraorbital angiolymphoid hyperplasia with eosinophilia. Australas Med J 2011;4:111–113 6. Kuo TT, Shih LY, Chan HL. Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 1988;12:843 7. Sun QF, Xu DZ, Pan SH, et al. Kimura disease: review of the literature. Intern Med J 2008;38:668–672 8. Buchdunger E, Cioffi CL, Law N, et al. Abl protein-tyrosine kinase inhibitor STI571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors. J Pharmacol Exp Ther 2000;295:139–145
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
595
A Mass Under Parotid Gland: A Patient With Kimura Disease To the Editor: Recently, we treated a 52-year-old middle-aged man with a mass under the left parotid gland. The size was equivalent to a soybean 3 months ago, constantly growing and reaching recently to a size of an egg yolk with considerable pain. After surgical intervention, the biopsy revealed Kimura disease (Fig. 1). Without a pathologic report, we can only exclude metastatic tumors, blood disease, and mycobacterium tuberculosis infection. This is the first case of Kimura disease diagnosed in our hospital, which reminds us to pay more attention to it. Hence, we did the following work. Kimura disease is a chronic inflammatory disease of unknown etiology with a good prognosis. Clinically, patients present with head and neck neoplasm, which tends to enlarge progressively, involving regional lymph nodes or parotid gland metastasis. This disease is mostly found in Asians, mostly young men (the patient in this case is a middle-aged man). The ratio of men-towomen prevalence is 6:1 to 10:1, seen in all ages. This disease is most common in unilateral head and neck subcutaneous mass. The lump often has a deeper location. It can involve large salivary glands, especially the parotid gland and the submandibular gland, and nearly half of all cases of regional lymph node enlargement.1 The pathogenesis of Kimura disease is still unclear. Some scholars suggest that it is a kind of immune-mediated inflammation disease associated with autoimmune factors.2 The pathogenesis includes the following: autoimmune dysfunction, allergy, tumor proliferation and insect bites, parasites, Candida infection, and the like.1 The disease may be associated with nephrotic syndrome, skin lichen amyloidosis, mouth ulcers, refractory hypertension, and other diseases,3 whereas some evidence of its pathogenesis may be associated with abnormal systemic immune response. Half of the patients have pruritic skin lesions, but this is not the main reason why most patients seek medical treatment. Most patients seek medical treatment because of the increasing size of the lump. The disease's most important criteria are biochemical abnormalities such as elevated eosinophil counts and elevated serum immunoglobulin E levels, whereas 12% to 16% of patients have proteinuria, lymph nodes mass, or lumps preceding kidney failure by several months.4 Clinical manifestations of the disease should be the craniocervical neoplastic inflammation with differential diagnosis including lymphoma, eosinophilic granuloma, histiocytosis, metastatic cancer, parasitic infections, allergic diseases, and cervical lymph node tuberculosis.5 Kimura disease's treatments include surgery, radiation therapy, and low-dose glucocorticoid therapy. After a simple partial tumor resection, there is a recurrence rate of 40%, which leads to the consideration of postoperative radiotherapy or hormonal therapy. The symptoms can be controlled by hormonal therapy alone, but subsequent hormonal dose tapering can induce relapse. Therefore, the addition of radiotherapy and immunosuppressive agents can give a better outcome.6 Recently, Sun et al7 proposed a treatment with imatinib because of its inhibitory effect on the proteintyrosine kinases platelet-derived growth factor receptor and c-kit gene reported in the hypereosinophilic syndrome. Sun et al7 and Buchdunger et al8 found a positive expression of c-kit gene and platelet-derived growth factor receptor-α in patients with Kimura disease as well and concluded that imatinib could therefore have a beneficial effect on Kimura disease.
FIGURE 1. Eosinophilic infiltration and proliferation of postcapillary venules can be seen. No evidence of malignancy or metastatic deposits is seen (100 magnification [A, B] and 400 magnification [C, D]).
Kimura disease has a rare occurrence. Most clinicians lack awareness of this disease, and this could easily lead to misdiagnosis. This disease should be considered in patients with craniocervical neoplasm, slow onset of disease, elevated eosinophils count, and serum immunoglobulin E in biochemical analysis, especially if the values are significantly increased. Afterward, proceed to a pathologic examination for confirmation.
Yanxin Gong, MM Department of Thyroid Surgery the Second Affiliated Hospital of Dalian Medical University Liaoning Province, China Yongfu Zhao, MD Department of Thyroid Surgery the Second Affiliated Hospital of Dalian Medical University Liaoning Province, China [email protected]
REFERENCES 1. Bonils P, Moya-Plana A, Badoual C, et al. Intraparotid Kimura disease. Eur Ann Otorhinolaryngol Head Neck Dis 2013;130:87–89 2. Hallam LA, Mackinlay GA, Wright AM. Angiolymphoid hyperplasia with eosinophilia: possible aetiological role for immunisation. J Clin Pathol 1989;42:944–949 3. Chartapisak W, Opastirkul S. Steroid-resistant nephrotic syndrome associated with Kimura's disease. Am J Nephrol 2002;22:38 4. Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14:486–488 5. Bangal SV, Chitgopekar RP, Gupta AK. Orbital extension of supraorbital angiolymphoid hyperplasia with eosinophilia. Australas Med J 2011;4:111–113 6. Kuo TT, Shih LY, Chan HL. Kimura's disease. Involvement of regional lymph nodes and distinction from angiolymphoid hyperplasia with eosinophilia. Am J Surg Pathol 1988;12:843 7. Sun QF, Xu DZ, Pan SH, et al. Kimura disease: review of the literature. Intern Med J 2008;38:668–672 8. Buchdunger E, Cioffi CL, Law N, et al. Abl protein-tyrosine kinase inhibitor STI571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors. J Pharmacol Exp Ther 2000;295:139–145
The Journal of Craniofacial Surgery • Volume 26, Number 2, March 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
595
